CAPLA

1. Dr Nilesh Wasekar
MD Medicine
DM Hematology
Consultant Hematologist Hcg Hospital Nashik

2.  Thrombosis and thrombocytopenia is a most difficult combination to evaluate and treat
 High level of suspicion and early intervention can save the life of the patient

3.  A 31-year-old lady,
 PNC [day 11]
 presented with Fever, Rash, Hypotension, ARF, Leucocytosis, Breathlessness, Drawsy
 Was on ionotropic support for hypotension
 On ventilator
 On MHD
 Her blood pressure was normal, no h/o joint pain, alopecia and she has no neurological
deficits

4. Right foot Left foot

5.  has a platelet count of 20k, haemoglobin concentration of 8.9 g/L and
peripheral blood film was normal except few schistiocytes
 Past h/o abortion in 2 TM
 Serum creatinine 3.4
 liver enzymes borderline Billirubin 5 Direct 4
 PT 25 APTT 58 [no correction after mixing]

6. DIC
 Thrombotic thrombocytopenic purpura/HELLP
 Heparin induced thrombocytopenia
CAPLA

8. LA
ACLA Positive

9. Evidence of involvement of three or more organs, systems, and/or tissues*
Development of manifestations simultaneously or in less than one week
Confirmation by histopathology of small vessel occlusion in at least one organ or tissue†
Laboratory confirmation of the presence of antiphospholipid antibodies (lupus anticoagulant
and/or anticardiolipin antibodies)‡
*Usually clinical evidence of vessel occlusions, confirmed by imaging techniques when appropriate. Renal involvement is defined by a 50% rise in serum creatinine,
severe systemic hypertension (>180/100 mm Hg). and/or proteinuria (>500 mg/24 hours).
†For histopathological confirmation, significant evidence of thrombosis must be present, although vasculitis may coexist occasionally.
‡If the patient had not previously been diagnosed as having an APS, the laboratory confirmation requires that the presence of antiphospholipid antibodies must be
detected on two or more occasions at least six weeks apart

10. Definite catastrophic APS
i. All 4 criteria present
Probable catastrophic APS
i. All 4 criteria, except only 2 organs, systems, and/or tissues involved
ii. All 4 criteria, except for the absence of laboratory confirmation of aPLs
iii. Diagnostic criteria 1, 2, and 4
iv. Diagnostic criteria 1, 3, and 4, with the development of a third event >1week
but within 1 mo of presentation, despite anticoagulation

11. The performance of a biopsy is not required
for diagnostic purposes although it is highly
recommended
14th International Congress on Antiphospholipid
Antibodies Task Force Report on Catastrophic
Antiphospholipid Syndrome

12. 69% are females
59% primary APS
26.9% from SLE
3.4% from lupus-like disease
In 49.1% of patients, CAPS was the first manifestation
of APS

13. Kidneys in 73.0%
Lungs in 58.9%
Brain in 55.9%
Heart in 49.7%
Skin in 45.4%

14. Infection
Pregnancy
Surgery
High Ferritin
Low D3
Withdrawal of IS/AC

15. Possible
ACLA
Combination FP less likely
B2GP : More sensitive
Repeat testing after 12 weeks advisable

16. How to treat it
How to anticoagulate with such
low platelet

17. IVIG 400 mg
/kg/day 2
days
MPS 250
OD for 3
days
PLEX
Platelet
improved to
35 on day 2
Started on
conventional
heparin 5000
BID
Day 4 PC
1.6 added
ecosprin
Digital
ischemia
improved

18. • Heparin fb
Warfarin
• Ecosprin
• Good backup
of ICU team
• Steroid/ivig/Pla
sma exchange
[77% S]
• HCQ
• Avoid Platelet
transfusion
• IS
Thrombocyto
penia
SIRS/CS
Anticoagulati
on
Supportive
Care
14th International
Congress on
Antiphospholipid
Antibodies Task
Force Report on
Catastrophic
Antiphospholipid
Syndrome

21. Give
corticosteroids and
IVIg to raise
platelet counts
rapidly to a safe
level (ie,>30,000–
50,000/μL).
–Start Treatment to
maintain platelet
counts in a safe
range when
corticosteroids are
tapered and the
effect of IVIg starts
to wear off.
–Do not give
anticoagulation, no
matter what the
platelet count, in
patients with life-
threatening
bleeding or
bleeding requiring
transfusion (World
Health
Organization
[WHO] grade
III/IV). Consider
a vena cava filter in
DVT patients.
–In all other ITP
patients (no
bleeding,
petechiae,
hematomas, stable
hemoglobin =
WHO grade 0/I/II),
consider
anticoagulation.
–With platelet
counts ≥ 50,000/μL
start standard-dose
therapeutic
anticoagulation.
–With lower
counts,<50,000/μL,
give half-standard
doses and increase
to full doses when
platelets rise to
≥50,000/μL.

22. If life
threatening
bleeding
Patient is
planned for
surgery

23.  RITUXIMAB
 ECULIZUMAB
 CYCLOPHOSPHAMIDE

25.  CAPLA is a rare diagnosis
 High level of suspicion is important
 Mortality is high
 Steroids+Plasma Exchange+IVIG+anticoagulation is the way to go in such cases
 Multidisciplinary management is important

26. Thank You